TY - JOUR
T1 - Clinical presentation and outcomes in patients with antiphospholipid syndrome-associated adrenal hemorrhage. A multicenter cohort study and systematic literature review
AU - Meade-Aguilar, José A.
AU - Figueroa-Parra, Gabriel
AU - Yang, Jeffrey X.
AU - Langenfeld, Hannah E.
AU - González-Treviño, Mariana
AU - Dogra, Prerna
AU - Bancos, Irina
AU - Moynagh, Michael R.
AU - Murad, M. Hassan
AU - Prokop, Larry J.
AU - Hanson, Andrew C.
AU - Crowson, Cynthia S.
AU - Duarte-García, Alí
N1 - Publisher Copyright:
© 2024 Elsevier Inc.
PY - 2024/3
Y1 - 2024/3
N2 - Background: Adrenal hemorrhage (AH) can occur in patients with antiphospholipid Syndrome (APS). We aimed to characterize the clinical manifestations, treatments, and outcomes of patients presenting with APS-associated AH (APS-AH) through a retrospective cohort and a systematic literature review (SLR). Methods: We performed a mixed-source approach combining a multicenter cohort with an SLR of patients with incident APS-AH. We included patients from Mayo Clinic and published cases with persistent positivity for antiphospholipid antibodies and presenting with AH, demonstrated by imaging or biopsy. We extracted demographics, clinical characteristics, laboratory findings, treatment strategies, and outcomes (primary adrenal insufficiency and mortality). We used Kaplan-Meier and Cox models for survival analysis. Results: We included 256 patients in total, 61 (24%) from Mayo Clinic and 195 (76%) from the SLR. The mean age was 46.8 (SD 15.2) years, and 45% were female. 69% of patients had bilateral adrenal involvement and 64% presented adrenal insufficiency. The most common symptoms at presentation were abdominal pain in 79%, and nausea and vomiting 46%. Hyponatremia (77%) was the most common electrolyte abnormality. Factors associated with primary adrenal insufficiency were bilateral adrenal involvement at initial imaging (OR 3.73, CI; 95%, 1.47–9.46) and anticardiolipin IgG positivity (OR 3.80, CI; 95%, 1.30–11.09). The survival rate at five years was 82%. History of stroke was associated with 3.6-fold increase in mortality (HR 3.62, 95% CI; 1.33–9.85). Conclusion: AH is a severe manifestation of APS with increased mortality. Most patients developed permanent primary adrenal insufficiency, particularly those positive for anticardiolipin IgG and bilateral adrenal involvement.
AB - Background: Adrenal hemorrhage (AH) can occur in patients with antiphospholipid Syndrome (APS). We aimed to characterize the clinical manifestations, treatments, and outcomes of patients presenting with APS-associated AH (APS-AH) through a retrospective cohort and a systematic literature review (SLR). Methods: We performed a mixed-source approach combining a multicenter cohort with an SLR of patients with incident APS-AH. We included patients from Mayo Clinic and published cases with persistent positivity for antiphospholipid antibodies and presenting with AH, demonstrated by imaging or biopsy. We extracted demographics, clinical characteristics, laboratory findings, treatment strategies, and outcomes (primary adrenal insufficiency and mortality). We used Kaplan-Meier and Cox models for survival analysis. Results: We included 256 patients in total, 61 (24%) from Mayo Clinic and 195 (76%) from the SLR. The mean age was 46.8 (SD 15.2) years, and 45% were female. 69% of patients had bilateral adrenal involvement and 64% presented adrenal insufficiency. The most common symptoms at presentation were abdominal pain in 79%, and nausea and vomiting 46%. Hyponatremia (77%) was the most common electrolyte abnormality. Factors associated with primary adrenal insufficiency were bilateral adrenal involvement at initial imaging (OR 3.73, CI; 95%, 1.47–9.46) and anticardiolipin IgG positivity (OR 3.80, CI; 95%, 1.30–11.09). The survival rate at five years was 82%. History of stroke was associated with 3.6-fold increase in mortality (HR 3.62, 95% CI; 1.33–9.85). Conclusion: AH is a severe manifestation of APS with increased mortality. Most patients developed permanent primary adrenal insufficiency, particularly those positive for anticardiolipin IgG and bilateral adrenal involvement.
KW - Adrenal hemorrhage
KW - Antiphospholipid syndrome
KW - Classification criteria
KW - Cohort study
KW - Mortality
KW - Systematic review
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U2 - 10.1016/j.clim.2024.109906
DO - 10.1016/j.clim.2024.109906
M3 - Article
C2 - 38244823
AN - SCOPUS:85183990810
SN - 1521-6616
VL - 260
JO - Clinical Immunology
JF - Clinical Immunology
M1 - 109906
ER -